Abstract

In 44 consecutive patients with systemic sclerosis (SSc), plasma concentrations of
von Willebrand factor (vWf) were higher than those of the vWf propeptide, but the
propeptide showed less variability within patient subgroups. Higher values of the
propeptide were observed in patients with early pulmonary involvement. A closer correlation
of the propeptide than of vWf to biochemical markers of activity was also evident.
Our results suggest that the propeptide, despite a shorter circulating half-time and
lower plasma concentrations than vWf, is more useful in the assessment of disease
activity in SSc.